Chordomas are rare malignant tumors growing from embryonic remnants of the chord. In 30% cases, they are located at the base of the skull (cranial chordomas). Most cases are registered at the age of 30 to 60. To confirm the diagnosis, the patients undergo the biopsy followed by histological examination. The compulsory step in chordoma treatment is surgical removal of the tumor. Also, the radiosurgery, proton therapy and chemotherapy can be used.
For a long time, the cranial chordoma used to be removed by craniotomy. The radicality of this method was low, and the rate of complications was high.
In modern surgery, this intervention is performed via transnasal (through the nose), or transoral (through the mouth) access, which allows reducing the injury significantly. The affected tissues are excised with microsurgical instruments without compromising the healthly brain structures. The manipulation precision is monitored using the endoscopic techniques.
When possible, the tumor is removed completely, with the capsule. However, most patients are diagnosed when the formation invaded the surrounding tissues, so surgeons must be limited to a partial resection.
The essence of radiosurgery is a single tumor irradiation with thin beams of radiation from a variety of angles. The main mass of energy is released precisely into the pathological focus, not spreading to the adjacent healthy tissues. High-dose exposure leads to destruction of the genetic apparatus of malignant cells and their gradual destruction. The procedure effects are manifested over the next 6 to 24 months.
The main indication for radiosurgery is residual postoperative tumors. This technique can be recommended as the only treatment method for small (less than 3 cm) or inoperable tumors.
Good results in patients with chordomas are produced by radiosurgical interventions using the Gamma Knife and Novalis. Both types of therapy are performed on an outpatient basis in one day.
Proton therapy is a type of radiotherapy upon which the pathological focus is affected by the beams of heavy particles, i.e. protons. Unlike x-rays, proton beams provide a maximum energy concentration in the final point of impact and substantially do not lose its when passing through healthy surface tissues. In comparison with traditional radiation, such treatment is safer and more effective.
The proton therapy is indicated to patients with chordomas in the postoperative period for destruction of residual tumor. Procedures are conducted daily for several weeks.
Anticancer drugs are used upon postoperative radiotherapy failure, or in the case of disease recurrence. The patients are prescribed such innovative drugs as tyrosine kinase inhibitors and platinum drugs.